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Pulmonary Capillary Hemangiomatosis

Pulmonary Hemangiomatosis


Presentation

  • When a patient initially presents only with pulmonary complications, it is not easy for clinicians to suspect vEDS.[ncbi.nlm.nih.gov]
  • At present, only eleven cases have been reported.[ncbi.nlm.nih.gov]
  • In the present case, capillaries infiltrated the alveolar and bronchial walls but not the vascular walls. No signs or symptoms of pulmonary hypertension or right heart hypertrophy/dilatation were present.[ncbi.nlm.nih.gov]
  • Pericardial defect is a rare anomaly that has a variable presentation. Prompt recognition and early surgical intervention are associated with a favorable outcome.[ncbi.nlm.nih.gov]
  • A 27-year-old female patient had presented progressing exertional dyspnea due to pulmonary hypertension.[ncbi.nlm.nih.gov]
Pathologist
  • Pathologists will find this book tremendously helpful when faced with difficult cases of interstitial lung disease or lung granulomas. It should be part of every surgical pathologist's library.[books.google.de]
  • We provide the largest case series to date and highlight the need for pathologists to have a high level of suspicion for PCH in patients with CTD.[ncbi.nlm.nih.gov]
  • Abstract This article provides an overview of pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH), two disorders that challenge clinicians, radiologists, and pathologists because they often mimic pulmonary arterial hypertension[ncbi.nlm.nih.gov]
  • The diagnosis can be made when pathologists see capillary overgrowth in a lung biopsy specimen.[phassociation.org]
Recurrent Chest Pain
  • Chest pain: may occur Treatment In histologically-proven [[Pulmonary Veno-Occlusive Disease]] (with or without pulmonary capillary hemangiomatosis) In response to pulmonary vasodilator therapy (mainly continuous intravenous epoprostenol, but also with[mdnxs.com]
Suggestibility
  • The cause of the venous obliteration was not identified but the occasional identification of phlebitis suggests this plays a role in venous damage in some cases.[ncbi.nlm.nih.gov]
  • Progressive pulmonary hypertension was documented by serial cardiac catheterizations, and pulmonary function studies showed changes suggestive of chronic pulmonary congestion.[ncbi.nlm.nih.gov]
  • These findings suggest that vascular involvement in PCH is responsible for the induction of pulmonary hypertension.[ncbi.nlm.nih.gov]
  • Chest CT revealed diffusely spread patchy ground-glass opacities sparing subpleural parenchymal areas suggesting the diagnosis of pulmonary veno-occlusive disease (PVOD).[ncbi.nlm.nih.gov]
Cryptorchidism
  • We report a case of an 18-year-old high school student, with a past history of cryptorchidism, presenting with recurrent pneumothorax. Routine laboratory findings were unremarkable.[ncbi.nlm.nih.gov]

Workup

  • The workup of a patient with suspected PAH is designed to confirm the diagnosis, exclude or confirm the presence of an underlying cause, and assess the severity of the disease.[clevelandclinicmeded.com]
Kerley A Lines
  • There were no Kerley-B lines or pleural effusion. The ECG showed right axis deviation and right ventricular hypertrophy with ST–T changes ( Figure 2 ).[circ.ahajournals.org]
Kerley B Lines
  • There were no Kerley-B lines or pleural effusion. The ECG showed right axis deviation and right ventricular hypertrophy with ST–T changes ( Figure 2 ).[circ.ahajournals.org]
Lymphocytic Infiltrate
  • Cases with PCH also all showed a mild interstitial lymphocytic infiltrate but there was no venulitis or infarction.[ncbi.nlm.nih.gov]
Ventricular Hypertrophy
  • At an autopsy, severe aortic stenosis and cardiomegaly with both left and right ventricular hypertrophy were noted.[ncbi.nlm.nih.gov]
  • ECG showing sinus tachycardia and partial right bundle-branch block with right ventricular hypertrophy. Figure 3.[circ.ahajournals.org]

Treatment

  • We describe a case of PH associated with limited SSc, where treatment with prostacyclin was complicated by pulmonary edema. Autopsy revealed PCH as the pathologic basis for the PH.[ncbi.nlm.nih.gov]
  • Diagnosis by pulmonary biopsy is essential for allowing appropriate treatment.[ncbi.nlm.nih.gov]
  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • After three months of treatment with imatinib, the chest X-ray and echocardiography findings improved, and the WHO-FC class was III. One year after, however, the PAH worsened again, and the patient died 2.6 years after the first diagnosis.[ncbi.nlm.nih.gov]
  • Four years before admission a diagnosis of breast cancer was made, and she underwent mastectomy plus radiation therapy and treatment with oral antiestrogens. The chest X-ray showed bilateral interstitial infiltrates.[ncbi.nlm.nih.gov]

Prognosis

  • Diagnosis of PCH is challenging in the setting of CDH and portends a poor prognosis.[ncbi.nlm.nih.gov]
  • The prognosis is poor and median survival was 3 years from the first clinical manifestation. Dyspnea and right heart failure are the most common findings of the disease.[ncbi.nlm.nih.gov]
  • Disease (PVOD) Primary Pulmonary Hypertension (PPH) Chronic Pulmonary Thromboemboli Pathology Some evidence that proliferation of thin-walled capillaries in PCH is histologic reaction to PVOD Clinical Issues Normal pulmonary capillary wedge pressure Prognosis[radiologykey.com]
  • Prognosis of this disease remains poor, and the only definitive treatment is lung transplantation.[ncbi.nlm.nih.gov]
  • Prognosis is poor, with an estimated mean survival of 36 months. 1 A 53-year old man, former smoker (39 pack-years), presented with worsening dyspnoea upon exertion and fatigue lasting 4 years.[archbronconeumol.org]

Etiology

  • We herein present a case of PCH diagnosed after lung transplantation with a focus on its etiology and a key to clinical diagnosis.[ncbi.nlm.nih.gov]
  • Families with multiple affected individuals with PCH suggest a heritable cause although the genetic etiology remains unknown. We used exome sequencing to identify a candidate gene for PCH in a family with two affected brothers.[ncbi.nlm.nih.gov]
  • Pulmonary capillary hemangiomatosis is a rare vascular proliferative disease of unknown etiology. The common clinical features are slowly progressive and finally fatal pulmonary hypertension. The clinical diagnosis is usually difficult.[ncbi.nlm.nih.gov]
  • Unlike most pathology textbooks, diseases are grouped on the basis of pathologic findings rather than etiology, mirroring the actual practice of surgical pathology.[books.google.de]
  • […] rare cases without recognizable causative background disease ( Am J Surg Pathol 2006;30:850 ) Epidemiology Very rare, mostly in adults Mean age 30 years, range 2-71 years Pathophysiology Chronic passive congestion may lead to capillary proliferation Etiology[pathologyoutlines.com]

Epidemiology

  • Articles include: Epidemiology of Pulmonary Arterial Hypertension, Pathology of Pulmonary Hypertension, Genetics of Pulmonary Arterial Hypertension, Diagnosis of Pulmonary Arterial Hypertension, Pulmonary Hypertension Owing to Left Heart Disease, Pulmonary[books.google.de]
  • Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models[books.google.de]
  • Consider diagnosis of "secondary PCH" or PCH-like changes for proliferations seen in association with other disorders, reserving "primary PCH" for extremely rare cases without recognizable causative background disease ( Am J Surg Pathol 2006;30:850 ) Epidemiology[pathologyoutlines.com]
  • Epidemiology Rare disorder Occurs mainly in children and young adults (12 reported cases) [[Pulmonary Veno-Occlusive Disease]], pulmonary capillary hemangiomatosis, and [[Idiopathic Pulmonary Arterial Hypertension]] may all represent various points on[mdnxs.com]
  • Epidemiology. Prognosis. Referral centre. Abstract Recently, our view of pulmonary hypertension has been changed by the significant progress made in understanding the pathobiology, epidemiology and prognosis of the disease.[revespcardiol.org]
Sex distribution
Age distribution

Pathophysiology

  • […] seen in association with other disorders, reserving "primary PCH" for extremely rare cases without recognizable causative background disease ( Am J Surg Pathol 2006;30:850 ) Epidemiology Very rare, mostly in adults Mean age 30 years, range 2-71 years Pathophysiology[pathologyoutlines.com]
  • The pathophysiological basis of the increase in pulmonary vascular resistance is hypertensive vascular disease in small arteries and pulmonary arterioles.[revespcardiol.org]
  • Pulmonary hypertension: Pathophysiology and clinical disorders. Baum GL, Crapo JD, Celli BR, Karlinsky JB, eds. Textbook of Pulmonary Diseases. Philadelphia, Pa: Lippincott-Raven; 1998. 1273-95. Wagenvoort CA.[emedicine.medscape.com]
  • Although the etiology and pathophysiology of PAH and PVH are distinct, they are similar in that both cause elevated PA pressure that inevitably leads to right ventricular (RV) dilation and remodeling, followed by RV failure and death if RV compensation[clevelandclinicmeded.com]

Prevention

  • Also very rarely they will continue to grow and eventually rupture....These growing hemangiomas become cavernous and may require surgery to prevent unwanted rupture. ...Read more See 1 more doctor answer 10 Is there collagen in capillary blood vessels[healthtap.com]
  • Diuretics - Usually oral tablets taken daily to prevent fluid retention. Atrial Septostomy - A hole is made between the right and left atriums in the heart the procedure is similar to having a right heart catheter done.[nzpah.org.nz]
  • While diuresis is indicated to prevent volume overload in most patients with PAH, diuretics should be used with caution.[clevelandclinicmeded.com]
  • Identification of GCN2 as new redox regulator for oxidative stress prevention in vivo. Biochem. Biophys. Res. Commun. 415, 120–124 (2011). 19. Fessel, J.P. et al.[nature.com]

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